[Autoimmune encephalitis, clinical, radiological and immunological data].
Revue Neurologique. 2013-02-01; 169(2): 142-153
DOI: 10.1016/j.neurol.2012.05.014
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1. Rev Neurol (Paris). 2013 Feb;169(2):142-53. doi: 10.1016/j.neurol.2012.05.014.
Epub 2012 Oct 23.
[Autoimmune encephalitis, clinical, radiological and immunological data].
[Article in French]
Aupy J(1), Collongues N, Blanc F, Tranchant C, Hirsch E, De Seze J.
Author information:
(1)Département de neurologie, CHU de Strasbourg, 3, avenue Molière, BP 426, 67091
Strasbourg cedex, France.
INTRODUCTION: Encephalitis is an inflammatory or infectious disease with an acute
or subacute presentation. Immunological abnormalities in serum can be found but
may be underdiagnosed. In several cases, a paraneoplastic origin with anti-neuron
antibodies is noted. In all cases, other auto-antibodies can be found with or
without any neoplastic mechanism.
OBJECTIVES: The aim of our study was to describe a clinical, radiological and
immunological cohort of patients with autoimmune encephalitis and suggest a
diagnostic and therapeutic algorithm.
PATIENTS AND METHOD: We performed a retrospective study in an immunological unit
of neurology. All patients with autoimmune encephalitis between March 2000 and
October 2009 were included. The clinical, imaging and immunological evaluations
were recorded for each patient.
RESULTS: Our cohort included 16 patients (eight men and eight women), mean age
45.3±10years. All patients had acute or subacute neuropsychological or
neuropsychiatric impairment and all patients but one had temporal lobe
dysfunction confirmed by cerebral MRI, PET or SPECT. Epilepsy was observed in 56%
of cases, extra-temporal lobe impairment in 50%, including sleep disturbances. A
cancer was found in only 25% (two small-cell lung cancers, one testis seminoma,
one non-small-cell lung cancer with Merckel cells cancer). Anti-neuron antibodies
were noted in 56% of cases (two with anti-voltage gate potassium channel complex
antibodies (ab), two with anti-NMDA-R ab, two with anti-glutamate acid
decarboxylase ab, one with anti-Ma2, two with anti-Hu ab and two remained
uncharacterized). Systemic antibodies were found in 50% (one anti-gangliosides,
one anti-SSA and one anti-DNA and four antinuclear ab uncharacterized, two
anti-TPO and two anti-phospholipids). All patients received immunomodulatory
treatments, including intravenous immunoglobulins (IgIV) and cancer was treated.
Five patients achieved complete recovery, partial improvement was observed in 10
patients and two patients died.
DISCUSSION: Despite clinical homogeneity at presentation, clinical outcome seems
to be different between patients with antibodies against neuronal surface
antigens and those with antibodies against intracellular antigens, which are more
likely refractory to immunotherapy and paraneoplastic. The frequency of
extra-temporal lobe impairment suggests that the term of limbic encephalitis
should be changed to autoimmune encephalitis.
Copyright © 2012 Elsevier Masson SAS. All rights reserved.
DOI: 10.1016/j.neurol.2012.05.014
PMID: 23099105 [Indexed for MEDLINE]